POEMS syndrome
General Information (adopted from Orphanet):
| Synonyms, Signs: |
Takatsuki syndrome Polyneuropathy - endocrinopathy - plasma cell dyscrasia Crow-Fukase syndrome PEP syndrome Osteosclerotic myeloma |
| Number of Symptoms | 23 |
| OrphanetNr: | 2905 |
| OMIM Id: |
|
| ICD-10: |
D47.7 |
| UMLs: |
C0085404 C1510415 |
| MeSH: |
D016878 |
| MedDRA: |
10053869 |
| Snomed: |
425657001 79268002 |
Prevalence, inheritance and age of onset:
| Prevalence: | No data available. |
| Inheritance: |
Unknown [Orphanet] |
| Age of onset: |
Adult Elderly [Orphanet] |
Disease classification (adopted from Orphanet):
| Parent Diseases: |
Chronic acquired demyelinating polyneuropathy
-Rare neurologic disease Hematological disease associated with an acquired peripheral neuropathy -Rare neurologic disease Peripheral neuropathy associated with monoclonal gammopathy -Rare neurologic disease Plasma cell tumor -Rare hematologic disease -Rare oncologic disease |
Symptom Information:
|
|
(HPO:0000112) | Nephropathy | Occasional [Orphanet] | 92 / 7739 | |||
|
|
(HPO:0000869) | Secondary amenorrhea | Frequent [Orphanet] | 42 / 7739 | |||
|
|
(HPO:0003134) | Abnormality of peripheral nerve conduction | Very frequent [Orphanet] | 38 / 7739 | |||
|
|
(HPO:0000771) | Gynecomastia | Frequent [Orphanet] | 53 / 7739 | |||
|
|
(HPO:0008373) | Puberty and gonadal disorders | Frequent [Orphanet] | 156 / 7739 | |||
|
|
(HPO:0008207) | Primary adrenal insufficiency | Occasional [Orphanet] | 26 / 7739 | |||
|
|
(HPO:0005978) | Type II diabetes mellitus | Frequent [Orphanet] | 68 / 7739 | |||
|
|
(HPO:0000821) | Hypothyroidism | Frequent [Orphanet] | 141 / 7739 | |||
|
|
(HPO:0010622) | Neoplasm of the skeletal system | Frequent [Orphanet] | 30 / 7739 | |||
|
|
(HPO:0011001) | Increased bone mineral density | Frequent [Orphanet] | 78 / 7739 | |||
|
|
(HPO:0001182) | Tapered finger | Occasional [Orphanet] | 93 / 7739 | |||
|
|
(HPO:0002240) | Hepatomegaly | Very frequent [Orphanet] | 467 / 7739 | |||
|
|
(HPO:0001744) | Splenomegaly | Very frequent [Orphanet] | 337 / 7739 | |||
|
|
(HPO:0002225) | Sparse pubic hair | Occasional [Orphanet] | 76 / 7739 | |||
|
|
(HPO:0011362) | Abnormal hair quantity | Frequent [Orphanet] | 92 / 7739 | |||
|
|
(HPO:0007400) | Irregular hyperpigmentation | Very frequent [Orphanet] | 72 / 7739 | |||
|
|
(HPO:0001006) | Hypotrichosis | Occasional [Orphanet] | 219 / 7739 | |||
|
|
(HPO:0001072) | Thickened skin | Very frequent [Orphanet] | 87 / 7739 | |||
|
|
(HPO:0100026) | Arteriovenous malformation | Frequent [Orphanet] | 38 / 7739 | |||
|
|
(HPO:0010701) | Abnormal immunoglobulin level | Very frequent [Orphanet] | 49 / 7739 | |||
|
|
(HPO:0001004) | Lymphedema | Frequent [Orphanet] | 62 / 7739 | |||
|
|
(HPO:0002716) | Lymphadenopathy | Very frequent [Orphanet] | 129 / 7739 | |||
|
|
(HPO:0040075) | Hypopituitarism | Very frequent [Orphanet] | 32 / 7739 |
Associated genes:
ClinVar (via SNiPA)
| Gene symbol | Variation | Clinical significance | Reference |
|---|